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OBJECTIVE: Atlantoaxial subluxation (AAS) is a frequent manifestation of rheumatoid arthritis (RA). The instability of the craniocervical junction caused by AAS is a potentially fatal condition and may require surgical treatment. Systemic manifestations associated with RA may increase the risk of perioperative complications. We evaluated the longterm mortality and its determinants in RA patients with AAS after cervical spine surgery. METHODS: A retrospective study of consecutive patients treated at Kuopio University Hospital between 1994 and 1998. Preoperative risk factors, neurological impairment using the Ranawat classification, perioperative course, functional outcome, and survival status were evaluated. RESULTS: During the study period 86 rheumatoid patients with AAS underwent cervical spine surgery. The mean followup time was 7.5 years (range 5.0-9.8). During the followup, 32 patients (37%) died. The mean survival time after surgery was 7.2 years (95% CI 6.7-8.0). Seven patients experienced postoperative complications. Age, AAS other than horizontal, and occurrence of complications were independent predictors of mortality. In two-thirds of the patients there was relief or decrease of pain, and the functional capacity improved. Neurological deficits subsided in 53% of cases. CONCLUSION: Patients with RA should be actively studied for AAS or other cervical instability, even when cervical symptoms are minor. Attention should be paid to perioperative management of these patients. Surgical treatment may not decrease the mortality of patients with RA, but it may result in more symptom-free life-years.